International Practices in Managing Preconception, Pregnancy and Childbirth in Women With Glanzmann Thrombasthenia: A Survey From the European Association of Haemophilia and Allied Disorders (EAHAD)

Abstract

BACKGROUND: Glanzmann thrombasthenia (GT) is an inherited platelet disorder resulting in severely reduced platelet aggregation and increased bleeding tendency. Pregnancy and childbirth in women with GT present significant challenges for both mother and child. OBJECTIVE: The aim of this study was to present the current clinical practices in pregnancy and childbirth in GT. METHODS: A survey on the management of the preconception phase, pregnancy and childbirth in women with GT was conducted by the European Association of Haemophilia and Allied Disorders. RESULTS: Thirty-seven responses from 18 countries were analysed. Almost all respondents recommended multidisciplinary preconception counselling, though 11% lack access to obstetrics and gynaecology services focused on bleeding disorders. Delay in conceiving was reported by 62%. Ninety-one percent of respondents indicated that they would screen for platelet alloimmunisation; however, the timing of screening varied. The recommended treatment to prevent postpartum haemorrhage (PPH) most often comprised tranexamic acid (TXA) and recombinant activated factor VII (rFVIIa) (25%) or TXA, rFVIIa and platelet transfusion (22%). The latter combination was also the most frequently recommended treatment to have on standby. Seventy-five percent would advise a mode of delivery as per obstetric indication and 69% would recommend to avoid neuraxial anaesthesia. Half of the respondents would add pharmacological thromboprophylaxis. CONCLUSION: There is general consensus on the importance of preconception counselling, the screening for platelet alloimmunisation and the preferred mode of delivery. Opinions regarding the treatment of PPH and the use of thromboprophylaxis vary. The next step is to improve guideline implementation in daily practice while working toward an international consensus-based guideline.